Ontology Term : Pfam:PF12972 Alpha-N-acetylglucosaminidase (NAGLU) C-terminal domain EMBL-EBI

Description  Alpha-N-acetylglucosaminidase, a lysosomal enzyme required for the stepwise degradation of heparan sulfate [1]. Mutations on the alpha-N-acetylglucosaminidase (NAGLU) gene can lead to Mucopolysaccharidosis type IIIB (MPS IIIB; or Sanfilippo syndrome type B) characterised by neurological dysfunction but relatively mild somatic manifestations [2]. The structure shows that the enzyme is composed of three domains. This C-terminal domain has an all alpha helical fold [3].
Namespace  Domain

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